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Introduction Intense endurance exercise is discouraged for patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), as it can increase the risk for adverse cardiac events as well as accelerate disease progression. While a sedentary lifestyle is also not advisable, there is currently no prospective data on the safety of physical activity for ARVC patients. To describe ARVC patients' average and peak ventricular arrhythmia burden – estimated from the prevalence of premature ventricular contractions (PVC) – measured during and after different exercise modalities and intensities, in context with cardiac and physiological load. Methods Twenty ARVC patients (8 f/12 m, age 48 ± 15 years, BMI 24 ± 3 kg/m2, resting PVC burden 4 ± 4%) with a heterozygous pathogenic/likely pathogenic plakophilin-2 (PKP-2) variant prospectively performed different exercises while monitored via 12-lead ECG. The order of modalities was randomized and participants were instructed to stop when surpassing perceived exertion of 15 (6-20 Borg scale). Resistance exercises included 2-min two-legged squats and single arm biceps curls (20 repetitions each) while endurance exercise included 5-min treadmill walking at comfortable speed and 3-min cycling bouts at a heart rate (HR) of 80, 100 and 120 bpm. Each activity was followed by a 10-min recovery period. Blood lactate concentration [La-] was assessed at the end of each cycling bout, with 4 mmol/L defined as a threshold to describe high-intensity exercise. Results No adverse events (including sustained ventricular tachyarrhythmia) nor premature terminations occurred. Average PVC burden during activities (including 5 min recovery) was lower for biceps curls compared with all other activities (p < 0.046), despite having the second highest level of perceived effort (13.8 ± 1.7 units). Biceps curls elicited ~20 mmHg (p < 0.001) lower peak systolic blood pressure and ~40 bpm peak lower HR (p < 0.001) compared with cycling at 120 bpm, despite similar perception of effort (12.9 ± 1.7, p = 0.818). Peak PVC burden during the different activities (highest PVC count in 1 min) ranged between 0–57% among participants and it was lower during the activities (5 ± 8%) than the subsequent 5-min recovery (8 ± 8%, p = 0.006). but no differences were detected between activities. Five patients (25%) presented [La-] that exceeded 4.0 mmol/L when cycling at 120 bpm. Discussion/Conclusion PVC burden, a marker for arrhythmogenicity, was generally higher during recovery than during exercise, with the smallest burden found during the exercise with a small muscle mass, despite high perceived exertion. Thus, such exercises might be better suited for training in ARVC patients. Generalized recommendations for a maximum HR of 120 bpm during exercise likely predispose a high number of ARVC patients to inadvertently perform high-intensity exercise, risking accelerating disease progression. Therefore, individualized exercise prescription should be recommended.

Introduction Patients suffering from arrhythmogenic right ventricular cardiomyopathy (ARVC) should avoid intense endurance exercise to reduce the risk of adverse cardiac events and disease progression. On the other hand, an active lifestyle might be preferable to a sedentary one, which also brings a host of complications. Evidence for safe levels of physical activity in ARVC, however, is scarce. This is study aimed to describe the ventricular arrhythmia burden - estimated as the prevalence of premature ventricular contractions (PVC) - of different exercise modalities and intensities on ARVC patients. Methods The pilot analysis includes four (1F, 33 ± 12 yrs, BMI 24 ± 4 kg/m2) ARVC patients harboring a pathogenic plakophilin-2 variant and carrying an implantable cardioverter-defibrillator performed different exercises while monitored via 12-lead ECG. The order of modalities was randomized and participants instructed to stop when surpassing perceived exertion of 15 on the Borg 6-20 scale. Resistance exercises included two-legged squats and single arm biceps curls (each with 20 repetitions and 2 min duration) while endurance exercise included 5 min of treadmill walking, 3 min cycling bouts at heart rate (HR) of 80, 100 and 120 bpm as well as cycling at 120 bpm with 2 additional min of active cool down. Blood lactate concentration was assessed at the end of the cycling bouts. Results No adverse cardiac event were noted and no exercise was terminated for medical reasons. During walking HR was 76 ± 8 bpm, whereas PVC burden was 11 ± 6% (range 4-18%). Cycling at 82 ± 4 bpm induced a PVC burden of 7 ± 5% (range 3- 14%), which increased to 13 ± 8% (range 3- 21%) at 93 ± 2 bpm and further to 16 ± 16% (range 7-37%) at 105 ± 3 bpm. In all three modalities the PVC burden was higher in the first 3 min of recovery than during the activity itself. Adding a 2 min active cool down increased the PVC burden to 25 ± 16% (range 6-45%). 2-legged squats performed at 101 ± 15 bpm had a PVC burden of 9 ± 12%, which increased to 19 ± 14% at recovery. One arm biceps curls at 75 ± 13 bpm had a PVC burden of 4 ± 3% during the activity and 9 ± 5% during recovery. Perception of effort varied widely when cycling at 80 bpm (range 6-12) or 100 bpm (range 8-14), but less so at 120 bpm (range 13-15). Blood lactate concentration when cycling at 120 bpm ranged between 2.2 and 3.5 mmol/L, typically associated with exercise in the “heavy” intensity domain. Discussion/Conclusion ARVC patients present a high, intensity-dependent PVC burden during exercise and short-term recovery. However, we observed widely different PVC burdens and perceived exertion at a given HR during different exercise modalities, which calls for personalized recommendations on physical activity. Exercises with small muscle mass seem to minimize the PVC burden and training the different muscles separately could be an interesting avenue to maintain physical fitness in ARVC patients.